Peripheral neuroectodermal sarcoma of soft tissue (peripheral neuroepithelioma): a pathologic study of ten cases with differential diagnosis regarding other small, round-cell sarcomas.

摘要: Abstract Peripheral neuroepithelioma of soft tissue belongs to the group peripheral neuroectodermal tumors (PNETs), but because its clinical, biological, and morphological characteristics, it differs from other small, round-cell sarcomas that appear in children (neuroblastoma) or thoracopulmonary region (Askin's tumor) bone (peripheral sarcoma bone). We report ten new cases such PNET variety, based on their histologic, immunohistochemical, electron microscopic findings. In all these cases, clinicopathologic correlations demonstrated high malignancy, with an ominous outcome nine cases. The mean age patients was 32.6 years there a clear male predominance (eight men, two women). Histologically, presence Homer-Wright rosettes is mandatory for diagnosis, being complemented positive immunohistochemistry several neural immunomarkers using paraffin-embedded material. Neuron-specific enolase, E-36, HNK-1, chromogranin markers proved be number (S-100 protein, synaptophysin, GFA neurofilaments [70 kilodalton]) were absent. Electron microscopy confirmed structures, both by scanning transmission microscopy.